This gene encodes an enzyme that sulfurates the molybdenum cofactor which is required for activation of the xanthine dehydrogenase (XDH) and aldehyde oxidase (AO) enzymes. XDH catalyzes the conversion of hypoxanthine to uric acid via xanthine, as well as the conversion of allopurinol to oxypurinol, and pyrazinamide to 5-hydroxy pyrazinamide. Mutations in this gene cause the metabolic disorder classical xanthinuria type II which is characterized by the loss of XDH/XO and AO enzyme activity, decreased levels of uric acid in the urine, increased levels of xanthine and hypoxanthine in the serum and urine, formation of xanthine stones in the urinary tract, and myositis due to tissue deposition of xanthine.
Immunogen Information
Immunogen
Recombinant fusion protein of human MOCOS (NP_060417.2).
Gene ID
55034
Swissprot
Q96EN8
Synonyms
MOCOSHMCSMCSMOSXAN2
Calculated MW
98 kDa
Observed MW
98 kDa
Applications
Reactivity
Human
Tested Applications
WB
Conjugation
Unconjugated
Dilution
WB 1:500-1:2000
Concentration
1 mg/mL
Storage Buffer
PBS with 0.02% sodium azide, 50% glycerol, pH7.3
Storage Instructions
Store at -20°C. Avoid freeze / thaw cycles.
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